Improvement of Lymphedema in IgG4-Related Disease with Oral Glucocorticoid Therapy

Abstract

An 81-year-old Japanese man with IgG4-related disease (IgG4-RD) presented with marked edema in the right lower leg. He was diagnosed with IgG4-RD with retroperitoneal fibrosis, hilar and mediastinal lymphadenopathy, elevated serum IgG4 levels, and IgG4-positive plasma cell infiltration in a mediastinal lymph node. Lymphatic scintigraphy revealed an obstruction corresponding to edema due to retroperitoneal fibrosis. Glucocorticoid therapy resulted in rapid improvement. Lymphedema due to IgG4-RD with retroperitoneal fibrosis is rare and no standard treatment exists. This case underscores the importance of considering IgG4-RD which is a potentially reversible cause of lymphedema.

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Cite

BibTeX RIS

BibTeX

@article{matsushita2026improvement,
  title = {{Improvement of Lymphedema in IgG4-Related Disease with Oral Glucocorticoid Therapy}},
  author = {Yuta Matsushita and Masataka Umeda and Hirokazu Kurohama and Tomoki Origuchi and Kanako Kojima and Yoshika Tsuji and Remi Sumiyoshi and Naoki Iwamoto and Atsushi Kawakami},
  journal = {Internal Medicine},
  year = {2026},
  month = aug,
  doi = {10.2169/internalmedicine.6044-25},
  url = {https://www.jstage.jst.go.jp/article/internalmedicine/65/15/65_6044-25/_article},
  note = {CC BY-NC-ND},
}

RIS

TY  - JOUR
TI  - Improvement of Lymphedema in IgG4-Related Disease with Oral Glucocorticoid Therapy
AU  - Yuta Matsushita
AU  - Masataka Umeda
AU  - Hirokazu Kurohama
AU  - Tomoki Origuchi
AU  - Kanako Kojima
AU  - Yoshika Tsuji
AU  - Remi Sumiyoshi
AU  - Naoki Iwamoto
AU  - Atsushi Kawakami
PY  - 2026
DA  - 2026/08/01
JO  - Internal Medicine
DO  - 10.2169/internalmedicine.6044-25
UR  - https://www.jstage.jst.go.jp/article/internalmedicine/65/15/65_6044-25/_article
AB  - An 81-year-old Japanese man with IgG4-related disease (IgG4-RD) presented with marked edema in the right lower leg. He was diagnosed with IgG4-RD with retroperitoneal fibrosis, hilar and mediastinal lymphadenopathy, elevated serum IgG4 levels, and IgG4-positive plasma cell infiltration in a mediastinal lymph node. Lymphatic scintigraphy revealed an obstruction corresponding to edema due to retroperitoneal fibrosis. Glucocorticoid therapy resulted in rapid improvement. Lymphedema due to IgG4-RD with retroperitoneal fibrosis is rare and no standard treatment exists. This case underscores the importance of considering IgG4-RD which is a potentially reversible cause of lymphedema.
SN  - 1349-7235
SN  - 0918-2918
ER  -